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Protein

Complement component C8 beta chain

Gene

C8B

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells.

GO - Biological processi

  • complement activation Source: ProtInc
  • complement activation, alternative pathway Source: UniProtKB-KW
  • complement activation, classical pathway Source: UniProtKB-KW
  • cytolysis Source: UniProtKB-KW
  • immune response Source: ProtInc
  • regulation of complement activation Source: Reactome
Complete GO annotation...

Keywords - Biological processi

Complement alternate pathway, Complement pathway, Cytolysis, Immunity, Innate immunity

Enzyme and pathway databases

BioCyciZFISH:ENSG00000021852-MONOMER.
ReactomeiR-HSA-166665. Terminal pathway of complement.
R-HSA-977606. Regulation of Complement cascade.

Names & Taxonomyi

Protein namesi
Recommended name:
Complement component C8 beta chain
Alternative name(s):
Complement component 8 subunit beta
Gene namesi
Name:C8B
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:1353. C8B.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • extracellular region Source: Reactome
  • extracellular space Source: Ensembl
  • extracellular vesicle Source: UniProtKB
  • membrane Source: ProtInc
  • membrane attack complex Source: UniProtKB-KW
Complete GO annotation...

Keywords - Cellular componenti

Membrane attack complex, Secreted

Pathology & Biotechi

Involvement in diseasei

Complement component 8 deficiency, 2 (C8D2)
Disease susceptibility is associated with variations affecting the gene represented in this entry.
Disease descriptionA rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.
See also OMIM:613789

Organism-specific databases

DisGeNETi732.
MalaCardsiC8B.
MIMi613789. phenotype.
Orphaneti169150. Immunodeficiency due to a late component of complements deficiency.
PharmGKBiPA25952.

Polymorphism and mutation databases

DMDMi20141201.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 32Sequence analysisAdd BLAST32
PropeptideiPRO_000002359133 – 541 PublicationAdd BLAST22
ChainiPRO_000002359255 – 591Complement component C8 beta chainAdd BLAST537

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi65 ↔ 1001 Publication
Glycosylationi70C-linked (Man)1 Publication1
Glycosylationi73C-linked (Man)1 Publication1
Disulfide bondi76 ↔ 1101 Publication
Disulfide bondi79 ↔ 1161 Publication
Glycosylationi101N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi122 ↔ 1331 Publication
Disulfide bondi127 ↔ 1461 Publication
Disulfide bondi140 ↔ 1551 Publication
Glycosylationi243N-linked (GlcNAc...)2 Publications1
Disulfide bondi378 ↔ 4031 Publication
Modified residuei418Phosphothreonine1 Publication1
Disulfide bondi503 ↔ 5501 Publication
Disulfide bondi505 ↔ 5211 Publication
Disulfide bondi508 ↔ 5231 Publication
Disulfide bondi525 ↔ 5341 Publication
Glycosylationi551C-linked (Man)1 Publication1
Glycosylationi554C-linked (Man)1 Publication1
Disulfide bondi557 ↔ 5901 Publication

Post-translational modificationi

N-glycosylated; contains one or two bound glycans. Not O-glycosylated.4 Publications

Keywords - PTMi

Disulfide bond, Glycoprotein, Phosphoprotein

Proteomic databases

PaxDbiP07358.
PeptideAtlasiP07358.
PRIDEiP07358.

PTM databases

iPTMnetiP07358.
PhosphoSitePlusiP07358.

Expressioni

Gene expression databases

BgeeiENSG00000021852.
CleanExiHS_C8B.
ExpressionAtlasiP07358. baseline and differential.
GenevisibleiP07358. HS.

Organism-specific databases

HPAiHPA023694.

Interactioni

Subunit structurei

Heterotrimer of 3 chains: alpha, beta and gamma. The alpha and gamma chains are disulfide bonded. Component of the membrane attack complex (MAC). MAC assembly is initiated by proteolytic cleavage of C5 into C5a and C5b. C5b binds sequentially C6, C7, C8 and multiple copies of the pore-forming subunit C9.2 Publications

Protein-protein interaction databases

BioGridi107193. 2 interactors.
STRINGi9606.ENSP00000360281.

Structurei

Secondary structure

1591
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Turni78 – 81Combined sources4
Beta strandi82 – 86Combined sources5
Beta strandi89 – 91Combined sources3
Beta strandi104 – 109Combined sources6
Beta strandi123 – 126Combined sources4
Turni128 – 130Combined sources3
Helixi136 – 138Combined sources3
Beta strandi141 – 143Combined sources3
Beta strandi146 – 149Combined sources4
Helixi172 – 176Combined sources5
Beta strandi177 – 179Combined sources3
Turni181 – 183Combined sources3
Beta strandi186 – 189Combined sources4
Beta strandi201 – 205Combined sources5
Beta strandi208 – 212Combined sources5
Beta strandi216 – 221Combined sources6
Beta strandi229 – 236Combined sources8
Helixi237 – 240Combined sources4
Turni256 – 258Combined sources3
Turni269 – 271Combined sources3
Beta strandi272 – 275Combined sources4
Helixi276 – 283Combined sources8
Beta strandi290 – 297Combined sources8
Beta strandi299 – 306Combined sources8
Beta strandi308 – 310Combined sources3
Helixi315 – 321Combined sources7
Helixi330 – 340Combined sources11
Beta strandi342 – 352Combined sources11
Beta strandi355 – 361Combined sources7
Helixi362 – 365Combined sources4
Turni366 – 369Combined sources4
Helixi372 – 378Combined sources7
Turni379 – 381Combined sources3
Turni404 – 408Combined sources5
Helixi409 – 412Combined sources4
Turni413 – 415Combined sources3
Beta strandi419 – 426Combined sources8
Helixi435 – 439Combined sources5
Beta strandi441 – 443Combined sources3
Helixi449 – 457Combined sources9
Beta strandi461 – 469Combined sources9
Helixi470 – 473Combined sources4
Turni476 – 478Combined sources3
Helixi482 – 499Combined sources18
Helixi502 – 504Combined sources3
Beta strandi513 – 517Combined sources5
Beta strandi520 – 524Combined sources5
Helixi532 – 534Combined sources3
Beta strandi536 – 541Combined sources6
Beta strandi557 – 563Combined sources7
Beta strandi584 – 588Combined sources5

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
3OJYX-ray2.51B55-591[»]
ProteinModelPortaliP07358.
SMRiP07358.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP07358.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini64 – 117TSP type-1 1PROSITE-ProRule annotationAdd BLAST54
Domaini120 – 157LDL-receptor class APROSITE-ProRule annotationAdd BLAST38
Domaini158 – 504MACPFPROSITE-ProRule annotationAdd BLAST347
Domaini505 – 535EGF-likeAdd BLAST31
Domaini545 – 591TSP type-1 2PROSITE-ProRule annotationAdd BLAST47

Sequence similaritiesi

Belongs to the complement C6/C7/C8/C9 family.Curated
Contains 1 EGF-like domain.Curated
Contains 1 LDL-receptor class A domain.PROSITE-ProRule annotation
Contains 1 MACPF domain.PROSITE-ProRule annotation
Contains 2 TSP type-1 domains.PROSITE-ProRule annotation

Keywords - Domaini

EGF-like domain, Repeat, Signal

Phylogenomic databases

eggNOGiENOG410IE7H. Eukaryota.
ENOG410Y2J1. LUCA.
HOGENOMiHOG000231146.
HOVERGENiHBG106489.
InParanoidiP07358.
KOiK03998.
OrthoDBiEOG091G07LK.
PhylomeDBiP07358.
TreeFamiTF330498.

Family and domain databases

InterProiIPR023415. LDLR_class-A_CS.
IPR002172. LDrepeatLR_classA_rpt.
IPR001862. MAC_perforin.
IPR020864. MACPF.
IPR020863. MACPF_CS.
IPR000884. TSP1_rpt.
[Graphical view]
PfamiPF00057. Ldl_recept_a. 1 hit.
PF01823. MACPF. 1 hit.
PF00090. TSP_1. 2 hits.
[Graphical view]
PRINTSiPR00764. COMPLEMENTC9.
SMARTiSM00192. LDLa. 1 hit.
SM00457. MACPF. 1 hit.
SM00209. TSP1. 2 hits.
[Graphical view]
SUPFAMiSSF57424. SSF57424. 1 hit.
SSF82895. SSF82895. 2 hits.
PROSITEiPS00022. EGF_1. 1 hit.
PS01209. LDLRA_1. 1 hit.
PS50068. LDLRA_2. 1 hit.
PS00279. MACPF_1. 1 hit.
PS51412. MACPF_2. 1 hit.
PS50092. TSP1. 2 hits.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P07358-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MKNSRTWAWR APVELFLLCA ALGCLSLPGS RGERPHSFGS NAVNKSFAKS
60 70 80 90 100
RQMRSVDVTL MPIDCELSSW SSWTTCDPCQ KKRYRYAYLL QPSQFHGEPC
110 120 130 140 150
NFSDKEVEDC VTNRPCRSQV RCEGFVCAQT GRCVNRRLLC NGDNDCGDQS
160 170 180 190 200
DEANCRRIYK KCQHEMDQYW GIGSLASGIN LFTNSFEGPV LDHRYYAGGC
210 220 230 240 250
SPHYILNTRF RKPYNVESYT PQTQGKYEFI LKEYESYSDF ERNVTEKMAS
260 270 280 290 300
KSGFSFGFKI PGIFELGISS QSDRGKHYIR RTKRFSHTKS VFLHARSDLE
310 320 330 340 350
VAHYKLKPRS LMLHYEFLQR VKRLPLEYSY GEYRDLFRDF GTHYITEAVL
360 370 380 390 400
GGIYEYTLVM NKEAMERGDY TLNNVHACAK NDFKIGGAIE EVYVSLGVSV
410 420 430 440 450
GKCRGILNEI KDRNKRDTMV EDLVVLVRGG ASEHITTLAY QELPTADLMQ
460 470 480 490 500
EWGDAVQYNP AIIKVKVEPL YELVTATDFA YSSTVRQNMK QALEEFQKEV
510 520 530 540 550
SSCHCAPCQG NGVPVLKGSR CDCICPVGSQ GLACEVSYRK NTPIDGKWNC
560 570 580 590
WSNWSSCSGR RKTRQRQCNN PPPQNGGSPC SGPASETLDC S
Length:591
Mass (Da):67,047
Last modified:January 23, 2002 - v3
Checksum:iB01722A6F2E9AFCE
GO

Polymorphismi

The sequence shown is that of allotype C8B B.

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_027649108E → K.Corresponds to variant rs12067507dbSNPEnsembl.1
Natural variantiVAR_012642117R → G in allotype C8B A. 2 PublicationsCorresponds to variant rs1013579dbSNPEnsembl.1
Natural variantiVAR_027650261P → L.Corresponds to variant rs12085435dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M16973 mRNA. Translation: AAA51862.1.
AK313382 mRNA. Translation: BAG36180.1.
AL121998 Genomic DNA. Translation: CAC18532.1.
CH471059 Genomic DNA. Translation: EAX06641.1.
BC130575 mRNA. Translation: AAI30576.1.
X04393 mRNA. Translation: CAA27981.1.
CCDSiCCDS30730.1.
PIRiA43071. C8HUB.
RefSeqiNP_000057.2. NM_000066.3.
XP_016857724.1. XM_017002235.1.
UniGeneiHs.391835.

Genome annotation databases

EnsembliENST00000371237; ENSP00000360281; ENSG00000021852.
GeneIDi732.
KEGGihsa:732.
UCSCiuc001cyp.5. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Web resourcesi

C8Bbase

C8B mutation db

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M16973 mRNA. Translation: AAA51862.1.
AK313382 mRNA. Translation: BAG36180.1.
AL121998 Genomic DNA. Translation: CAC18532.1.
CH471059 Genomic DNA. Translation: EAX06641.1.
BC130575 mRNA. Translation: AAI30576.1.
X04393 mRNA. Translation: CAA27981.1.
CCDSiCCDS30730.1.
PIRiA43071. C8HUB.
RefSeqiNP_000057.2. NM_000066.3.
XP_016857724.1. XM_017002235.1.
UniGeneiHs.391835.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
3OJYX-ray2.51B55-591[»]
ProteinModelPortaliP07358.
SMRiP07358.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107193. 2 interactors.
STRINGi9606.ENSP00000360281.

PTM databases

iPTMnetiP07358.
PhosphoSitePlusiP07358.

Polymorphism and mutation databases

DMDMi20141201.

Proteomic databases

PaxDbiP07358.
PeptideAtlasiP07358.
PRIDEiP07358.

Protocols and materials databases

DNASUi732.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000371237; ENSP00000360281; ENSG00000021852.
GeneIDi732.
KEGGihsa:732.
UCSCiuc001cyp.5. human.

Organism-specific databases

CTDi732.
DisGeNETi732.
GeneCardsiC8B.
HGNCiHGNC:1353. C8B.
HPAiHPA023694.
MalaCardsiC8B.
MIMi120960. gene.
613789. phenotype.
neXtProtiNX_P07358.
Orphaneti169150. Immunodeficiency due to a late component of complements deficiency.
PharmGKBiPA25952.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IE7H. Eukaryota.
ENOG410Y2J1. LUCA.
HOGENOMiHOG000231146.
HOVERGENiHBG106489.
InParanoidiP07358.
KOiK03998.
OrthoDBiEOG091G07LK.
PhylomeDBiP07358.
TreeFamiTF330498.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000021852-MONOMER.
ReactomeiR-HSA-166665. Terminal pathway of complement.
R-HSA-977606. Regulation of Complement cascade.

Miscellaneous databases

EvolutionaryTraceiP07358.
GenomeRNAii732.
PROiP07358.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000021852.
CleanExiHS_C8B.
ExpressionAtlasiP07358. baseline and differential.
GenevisibleiP07358. HS.

Family and domain databases

InterProiIPR023415. LDLR_class-A_CS.
IPR002172. LDrepeatLR_classA_rpt.
IPR001862. MAC_perforin.
IPR020864. MACPF.
IPR020863. MACPF_CS.
IPR000884. TSP1_rpt.
[Graphical view]
PfamiPF00057. Ldl_recept_a. 1 hit.
PF01823. MACPF. 1 hit.
PF00090. TSP_1. 2 hits.
[Graphical view]
PRINTSiPR00764. COMPLEMENTC9.
SMARTiSM00192. LDLa. 1 hit.
SM00457. MACPF. 1 hit.
SM00209. TSP1. 2 hits.
[Graphical view]
SUPFAMiSSF57424. SSF57424. 1 hit.
SSF82895. SSF82895. 2 hits.
PROSITEiPS00022. EGF_1. 1 hit.
PS01209. LDLRA_1. 1 hit.
PS50068. LDLRA_2. 1 hit.
PS00279. MACPF_1. 1 hit.
PS51412. MACPF_2. 1 hit.
PS50092. TSP1. 2 hits.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiCO8B_HUMAN
AccessioniPrimary (citable) accession number: P07358
Secondary accession number(s): A1L4K7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: April 1, 1988
Last sequence update: January 23, 2002
Last modified: November 30, 2016
This is version 170 of the entry and version 3 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.